Renal Medicine Flashcards
1
Q
What is the presentation for nephritic syndrome?
A
Haematuria, proteinura (<200), hypertension, renal impairment, peripheral oedema
2
Q
What are the 3 types of nephritic syndrome?
A
Post-strep GN
Chronic GN (FSGS, MPGN, membranous GN, IgA N, SLE, HSP)
RPGN (cresenteric)
3
Q
What do the complement levels do in post-strep GN?
A
Low C3, NORMAL C4 (Strep is 3x more dangerous)
4
Q
What do the C3 and C4 levels do in MPGN?
A
PERSISTENLY LOW C3. BUT ALSO LOW C3 and C4 to start with
5
Q
What conditions causing nephritis will have low C3 and C4?
A
MPGN, shunt nephritis, SLE
6
Q
What is the most common cause of nephritic syndrome?
A
IgA nephropathy (different to IgA vasculitis ie HSP. Most common type of vasculitis in children)- post infection
7
Q
What is the treatment for IgA nephropathy?
A
Immunosuppression, needs atleast 6 months of steroids.
ACEI for persistent proteinuria
8
Q
What conditions have a NORMAL complement in nephritis?
A
FSGS, IgA nephropathy, HSP, ANCA, HUS, ALports (alports has SSNL)
9
Q
How do you calculate the FENa?
A
URINE Na x serum Cr x 100
DIVIDED BY
PLASMA sodium x urine Crr ratio
10
Q
How much should your BP dip by overnight?
A
10%
11
Q
Anterior lenticonus is pathognomonic of which suyndrome
A
Alports.
Present in 30%
12
Q
What is a risk of NF in relation to the kidneys
A
Renal artery stenossi
13
Q
What are the three embryological origins of the kidney
A
Pronephros
Mesonephros - functioning kidney until week 10. Helps form ureters and bladder, later becomes gonads
Metanephros - this form the actual kidney
14
Q
What structure forms the foetal kidney and what time in the embryological period is this
A
Mesonephros
Weeks 5-10
15
Q
What structures forms ACTUAL kidney
A
Metanephros
MET criteria for actual kidney
16
Q
When does the pronephros appear
A
4 weeks
17
Q
When does renal development stop in the embryogenic period
A
36 weeks OR 4 weeks postnatal period which ever comes first
18
Q
When does the amniotic fluid become predominantly urine
A
15 weeks
Before that, 2/3 urine and 1/3 pulmonary fluid
19
Q
Does anhydromnios always mean renal dysfn
A
No, can be invivo placental dialysis, these kids will have normal renal funtion in later life
20
Q
AT birth, what is the GFR % compared to adult and when does the GFR reach adult values
A
5% at birth, takes 2 years to reach adult levels
21
Q
What is normal FENa (%)
A
1%
22
Q
Why do premature infants have a high FENa
A
Premature kidneys so often need sodium supplementation to avoid hyponatraemia
23
Q
Between what time frame is there a rapid increase in GFR post birt
A
First 4 days
24
Q
HOw long does it take for the GFR to double post birth
A
2 weeks.
2x by 2 weeks
25
What is the most metabolic part of the kidney
PCT
26
What is the name of the transporter responsible for Na reabs in PCT
Na-K ATPase-active transporter
27
What is resorbed in the PCT
Na, Bicarb, Phos, Glu, Ca (basically everything ex Mg which is MOSTLY absorbed in LOH)
28
What is Fanconi syndrome
Proximal tubule dysfn- phophaturia, glucosuria, hypouricaemia, amonoaciduria
29
Clinical presentation of Fanconi syndrome
FTT, polyuria, polydipsia, dehydration, constipation, ricketts.
30
Most common genetic cause of Fanconi syndrome
Cystinosis
31
What type of RTA does Fanconi syndrome present with
Type 2 RTA- proximal. HCO3 wasting.
Fanconi is TWICE as worse.
32
Main function of Loop of Henle
Na, Ca, Mg and K resorption
33
How is sodium resorbed in the loop of Henel
Na K Cl2 transporter
34
Where do loop diuretics bind and how does it change BP
Bind and INHIBIT Cl on the NAKCL2 transporter, means no Na resorbed and so no water resorbed.
In Barter's there is an inborn error of metabolism in the Cl part of this channel
35
How is Na resorbed in the distal tubule
Na Cl co-transporter
36
Where is the main site of aldosterone action
collecting tubule and then DCT
37
Where is Gitelmann syndrome affected
DCT
38
What does Aldosterone do in the Collecting tubule
increase ENaC and NaK ATPase activity to increase water and Na resorption
39
How does Spiro work
Blocks aldosteroner receptors so CANNOT increase ENAC and NaK ATPase to increase water resorp. K sparing.
40
What does ADH do in the colleting duct
Increase release of Aquaporin from the vesicles to increase water resorption
41
What releases renin
Juxtaglomerular appratus
42
What are the triggers for renin release
THINK END GOAL IS TO RESORB WATER.
Stretch receptors (Reduced in afferent), baroreceptors and sympathetic activity, increased solute load
Ahhhhhh = sounds of a nice stretch= afferent
43
Name the pathway of renin-angiotensin-aldosterone system
REnin from juxtaglomerular apparatus activates angiotensin (from liver) to angiotensin I which --> Angiotensin II with ACE.
This causes systemic vasoconstriction and increase aldosterone release.
44
What is Conns syndrome
primary hyPER aldosteronism- so hypertension, raised ECF volume expansion and renin suppression
45
What is Liddle syndrome
AD disorder- gain of function in ENAC Channel
increased sodium and water resorption but renin AND also suppressed on bloods
46
Treatment for Liddle syndrome
Amiloride- blocks ENaC channels
47
What medication blocks ENAc channels
Amiloride
48
What cells repease EPO
Peritubular cells in the renal cortex
INTERSTITIAL FIBROBLASTS.
49
Action of 1-hydroxylase
Convert 25-OHVitD to 1,25-OHVit D
50
What gives a false neg in terms of haematuria
Vit C/ascorbic acid
51
Are nitrites specific or sensitive for UTI
Specific
52
What scan is good for VUR
MCUG- dye from the bladder UP
Also good for posterior urethral valves
53
What is DMSA scan good for?
STATIC scan- just a picture.
Good for UTI, ectopic or duplex kidney
54
What is MAG3 good for
Dynamic scan- isotope filtered from kidneys down to bladder. Good for upper urinary tract obstruction.
55
Most common cause of CKD in kids?
CAKUT
56
What does a PUJ obstruction lead to ? And what is a scan used for further assessment for this?
Hydronephrosis
MAG 3- REMEMEBER UPPER TRACT PROBLEMS
57
What shows up on a DMSA scan for multicystic dysplastic kidney
No functioning kidney
58
What are associations of Horseshoe kidney
Turners
Trisomy 21
VACTERYK
Fanconi Anaemia
59
What surgical procedure should NOT be performed in babies with hypospadias
circumscision
60
3 common examination findings of testicular torsion
horizontal lie, loss of cremasteric reflex and diffuse tenderness
61
Where is the pain in torsion of hyatid of Morgagni
Upper pole of testes, common in prepubertal age
62
Name 5 genetic causes of Fanconi syndrome
1. Cystonosis
2. Galactossemia
3. Tyrosinaemia
4. Hereditary fructose intolerance
5.- Glycogen storage disease
6. Lowes syndrome
63
What is Lowe's syndrome
Mental retardation
Reduced tone
renal dysfn
64
What type of disorder is CYtininsos
Glycogen storage disorder- defect in gene that codes for cystine transport protein.
65
Difference between cystinosis and cystinuria
Cystinuria= defect in reabs of and hence excessive excretion of amino acids = recurrent stone formation.
Cystinosis= defect in carrier --> deposition in eyes, kidneys etc
66
Where is the problem in x linked hypophos rickets
In PCT- prob with reabs of phosphate
67
Electrolyte abnormalities in Barters syndrome
Low Cl
Low K
Mg, Ca and Na may also be low
68
What is in the urine for Barters
INAPPROPRIATELY HIGH urine Cl and NA. Also calcium.
69
Difference between pseudo and actual Barters?
Pseudo will have appropriately low urine Cl and Na
70
What can cause pseudo Barters
THINK LOW Cl
- congenital chloride diarrhoea
- CF
- laxative abuse
71
Which drug/class of drugs have the same effect as Barters
Loop diuretics ie Frusemide
72
Differentials of HYPOkalemic, HYPOchloremic metabolic alkalosis in kids
1. Surreptious vomiintg (hidden)
2. Laxative abuse/ Congenital Cl losign diarhoea
3. Diuretic abuse
73
The epithelial sodium channel in the collecting duct is regulated by what? (past question)
ENaC
Aldosterone.
74
What does Gittelmans present with
Hypokalemia
Met alkalsois
Hypomagnesemia
HyPOcalciuria
75
What is the location of Type 1 bs Type 2 RTA
Keep 2 things closer so Type 2 is proximal and type 1 is distal
76
Which RTA has low bicarb
Type 1 (Distal) BUT proximal harder to treat so supplement with bicarb
77
Features of Type 1 RTA- clinical
Nephrocalcinosis, faltering growth, severe hypokalemia
78
What condition can trimethoprim and toulene (glue) cause
Type 1 RTA
79
Main defect in type 1 RTA
Failure of H+ excretion so no bicarb abs
80
Which condition has idiopathic hypercalcemia of infancy
Williams syndome
81
What do symptomatic renal cysts present wit
Symptomatic simple renal cysts may present with loin pain, haematuria, or recurrent UTI.
82
What is the most common outcome of a MCDK in the long term
Normal renal function (overall)
83
What are some common complicatiosn of MCDK
15% have contralateral VUR
5-10% have contralateral hydronephrosis
0.2-1.2% develop hypertension
0.3% risk of Wilms
84
How much %body weight is water in preterm vs term babies
85% in preterm
80% in term
85
What is a safe rate of correction for hyponatraemia
1mmol/4 hours
Total 6-8mmol/24 hours
86
In conditions with hyPOkalemia, what happens to Cl in urine
LOW to compensate. To do with the transporters.
87
Medication to treat hyperkalemia - the most important
Calcium gluconate- stabilise myocardium
88
Medication to treat hyperkalemia post stabilisation of myocardium
insulin-dex
salbutamol
dialyssi
89
4 main symptoms of hyPOcalcaemia
Tetany, paresthetias, muscle cramps, seizures
90
Main symptoms of HyPERcalcaemia
Moans, groans, bones and stones
Headache, constipation, lethargy, renal stones, hallucinations
91
What is more accurate when testing for protein loss- PCR or ACR
PCR more accurate (think PCR more accurate in COVID)
92
What is the most common cause of proteinuria in kids
Benign orthostatic proteinuria
93
Which nephritic syndromes are caused by type 3 hypersensitivity reactions
IgA nephropathy
Post-strep GN
94
Which nephritic syndrome is caused by type 2 hypersensitivity reactions
Goodpastures
95
What is the definition of nephritic range proteinuria
>200g/L
96
What are the three types of RPGN
Goodpastures- type 2
ANCA associated GPA or microscopic polyangitis
97
Which RPGN has upper airway involvement
GPA or any ANCA associated vasculitis
(pulmonary renal syndrome)
98
Name the disease:
Recent sore throat. Low C3
PSGN
99
Recent illness weeks ago. Spots on back of legs and buttocks. Joint pain.
HSP
100
Haemturia post URTI
Ig A nephropathy
101
Pulmonary renal syndrome associated with what autoimmune marker?
*Hint think of a disease that affects both*
ANCA associated
102
Persistently low C3
Membranioproliferative
103
Most common nephrotic syndrome
Minimal change
Nephrotic does not care about change.
104
3 diagnostic crteria for nephrotic syndrome
Proteinuria (>200), hypoalbuminaemia and oedema
105
Age for atypical nephrotic syndrome
<18months or >12yo
106
What is the definition of remission in nephrotic syndrome
>3 days of no proteinuria
107
Treatment for nephrotic? (First line)
Steroids: 60mg/m2 for one month with prophylactic antibiotics
108
Treatment for frequently relapsing nephrotic syndrome
Calcineurin inhibitor- Cyclosporin or Tacro. Tacro more fabourable
109
What class of drugs is Tacrolimus
Calcineurin inhibitors
110
What is the natural history of nephrotic syndromes
Gets better once you hit puberty
111
What % of kids respond to steroids in nephrotic syndrome
90%
112
What % of kids will have a relapse in nephrotic
75%
113
What percentage of kids will have multiple relapses in nephrotic
50%
114
What subset of kids with nephrotic are most likely to grow out of their nephrotic syndromes- ie the name given to this group
Steroid sensitive
115
What are known complications of nephrotic syndrome (name 5)
Thrombosis (saggital sinuts thrombosis), Vit D def, hypothyroidism, raised chol, infection (due to oedema)
116
Dilatation of the afferent glomerular arteriole to maintain GFR during times of hypotension, is mediated by which of the following?
PGE2
117
What medication is an absolute contraindication in phaechromocytoma?
Beta blockers. Because unopposed alpha action which can cause MI and brain bleeds.
118
What is the most appropriate dietary management to reduce renal stones
reduce sodium
119
In nephrotic syndrome hypercoagulability is due to deficiency of
Antihrombin 3
120
Growth hormone is used in chronic renal disease. What is the mechanism of GH deficiency in chronic renal disease?
GH resistance
121
Haemofiltration is a process best described by
ULTRAFILTRATION
remember peritoneal dialysis= osmotic pressure
122
What is the medication used for raised acute HTN causing neurovascular changes ie PRESS
Sodium nitroprusside is a medication used in the management of acute hypertension. It is a potent vasodilator and is administered as an IV infusion with intensive monitoring in place
123
Primary mechanism leading to oedema in nephrotic syndrome
Reduced capillary oncotic pressur
124
Relationship between Angiotensin II and Na?
In the proximal convoluted tubule of the kidney, angiotensin II acts to increase Na-H exchange, increasing sodium reabsorption. Increased levels of Na in the body act to increase the osmolarity of the blood, leading to a shift of fluid into the blood volume and extracellular space (ECF).
125
What forms the collectinf systems of the kidneys
Ureteric bud
126
What mediates renal efferent vasoconstriction
Angiotensin II
127
What is primarily activated in PSGN (ie which complement pathway)
ALternate pathway
Remember only C3 low
128
Treatment for hypocalcaemia in an ED setting.
Ca gluconate
129
What should you correct first, hypocalcaemia or acidosis
Acidosis as Calcium depends on pH
130
What values of FENa determine pre-renal vs renal cause of AKI
<1%= pre-renal
>2%= ATN
131
Name 5 complications of AKI
Acidosis
Hyperkalemia
Hypocalcemia
Severe symptomatic uraemia
Fluid overload
132
What happens to ADAMS T13 in HUS
LOW
133
Most common cause of CKD in kids (1 and 2)
1. CAKUT
2. Chronic glomerulonephritis
134
What are indications to start dialysis
Hyperkalemia - refractory to treatment
Uremia
Drug OD ie vanc/gentamyxin
Fluid overload
Acidosis
High phosphate
135
Name what grade VUR this is.
Reflux into non dilated ureter
Grade 1 (pic in notes)
136
Name what grade VUR this is.
Into pelvis and calyces without dilatation
Grade 2
137
Mild to mod dilatation of ureter, renal pelvis and calyces with minimal blutning of the fornices
Grade 3
138
Moderate ureteral tortuosity and dilatation of the pelvis and calyces
Grade 4
139
Gross dilatation of the ureter, pelvis and calyces with loss of papillary impressions and ureteral tortuosity
Grade 5 VUR
140
In kids with UTI, who should have an ultrasound prior to discharge
Seriously unwell children, those with renal impairment, and boys <3 months of age should have a renal ultrasound prior to discharge
141
Who should get non-urgent renal ultrasounds post UTI
Only kids with recurrent UTIs
142
What two virus can cause cystitis
Adenovirus and Influenza
143
When do most children become dry in the day vs at night
At night- 6-9 months and daytime bu 3 years
144
Definition of primary nocturnal eneursis
Never achievement night time dryness
145
What is the most common cause of daytime voiding disorder
Overactive bladder and URGE = most common symptom
146
What is the expected bladder capacity
(age+1) x 30 mls
147
When should Desmopressin be used in nocturnal eneurisis
FOr special situations ie sleepover.
Only good for short term
148
What is the medication for detrusor instability
Oxybutynin
(anti-cholinergic)
149
Classic presentation of voiding postponement
Delay going to the toilet when participating in activities/at school etc --> overflow incontinence.
150
Classic presentation of underactive bladder
Decreased voiding frequency secondary to neurological causes ie sacral agenesis, bladderoutlet obstruction. Need to optimise bladder emptying and pelvic floor relaxation
151
What is dysfunctional voiding
Incomplete relaxation of pelvic floor muscles and during bladder emptying
152
What is vaginal reflux
Entrapment of urine by labia because of poor posture/entrapment by thighs (overweight).
Leads to wetting after voiding/standing up/physical activities when trapped urine leaks out.
153
What is giggle incontinence
Rare often hereditary. Involuntary complete ballder emptying on laughing with otherwise normal bladder and bowel function
154
What is extraordinatory day time frequency incontinence
Isolated sudden frequent voiding of small volumes without nocturia, atleast 1/hour. Usuaull self limited. Can try anticholinergic.
155
What condition:
Flank mass and HTN
Wilms
156
What condition:
Cafe au lait and HTN
NF1 - assocaited with renal artery stenosis
157
What condition:
Tachycardia, flushed, HTN
Phaechromocytoma
158
Which is worse, aut recessive or dominant PKD
RECESSIVE
159
US findings for ARPKD
large, echobright kidneys. Wont see true cysts
160
What other condition is ALWAYS associated with ARPKD
Hepatic fibrosis
161
What other condition is ALWAYS associated with ARPKD
Hepatic fibrosis
162
Gene for ARPKD vs ADPKD
PKHD1 mutation
6 for recessive
PKD1
16 for dominant
(Dominant larger)
163
US for ADPKD
Discrete cysts
164
Classic appearnce of MCDK
multiple non communicating cysts with non functioning renal parenchyma- only one sided.
Should always monitor other kidney
165
Main cause of nephrocalcinosis
Distal RTA - remember thats type 1 RTA
166
3 other causes of nephrocalcinosis
Ex-prem neonates
Vit D treatment for hypophosphataemic rickets
oxalosis
167
Most common renal stone type
Calcium oxalate
168
Most common kidney stone disorder?
Cystinuria (NOT CYSTINOSIS)
Defect in renal reabs of cystine
169
Mechanism of action of acetazolamide
Acetazolamide= Carbonic ANHYDRASE inhibitor
Prevents reabs of sodium and bicarb
170
What does Dopamine do to afferent and efferent arterioles
DILATATION OF BOTH.
Increased renal blood flow to both but no increase in GFR
171
What is Dent Disease
Thing about going to the dentist.
Dent disease is a rare genetic kidney disorder characterized by spillage of small proteins in the urine, increased levels of calcium in the urine, kidney calcifications (nephrocalcinosis), recurrent episodes of kidney stones (nephrolithiasis) and chronic kidney disease. Dent disease affects males almost exclusively.
There is a guy driving a car who has kidney stones and calcifications due to high Ca, DENTs his car and results in leaky protein post.
172
What is medullary sponge kidney disease
Medullary Sponge Kidney is a rare disorder characterized by the formation of cystic malformations in the collecting ducts and the tubular structures within the kidneys (tubules) that collect urine. One or both kidneys may be affected. The initial symptoms of this disorder may include blood in the urine (hematuria), calcium stone formation in the kidneys (nephrolithiasis) or infection. The exact cause of Medullary Sponge Kidney is not known.
173
Common presentation of uraemia or CKD in kids
Vomiting
174
How to calculate anion gap
Positive- negative
K+Na - Cl+HCO3-
175
First and second largest site for NA reabs
1. PCT
2. Thick ascending loop of Henle
176
Meds causing Fanconi syndrome
Aminoglycoside, vlaproatem cisplatin, ifosfamide
(think of the onc drugs causing nephropathy)
177
Which RTA has risk of hypercalcemia and stones
Type 1 RTA
TYpe 1 NUMBER 1 cause of hypercalcemia
178
What is type 4 RTA
HyPOaldosteronism
179
What happens to K in Type 1, 2 and 4 RTA
Type 1 and 2: LOW
Type 4: HIGH
180
Triggers for aldosterone release
Angiotensin II
Hyperkalemia
181
Which condition- barter or gitelmans has hypomagnesemia?
GIttelMan - Low Magnesium
Barters has high ruinary cl and nephrocalcinosis due to calciuria
182
Which medication does Gittelman replicate
thiazide diuretic
183
What medication used for intracranial HTN causes hypercalciuria
Acetazolamide
184
What is the term given to this presentation in the setting of prev nephrotic syndrome?
3 consecutive early morning urine samples with 3+ protein
Relapse
185
What is the term given to this presentation?
Initially responsive to steroids but 2 relapses in 6 months of initial response
Frequently relapsing nephrotic
186
What is the term given to this presentation?
Relapsing nephrotic while on steroids
Steroid dependency
187
What is the term given to this presentation?
No response after 4 weeks of steroid therapy
Steroid non-responder or steroid resistant
188
First and second mot common type of Nephrotic syndrome
1. Minimal change
2. FSGS
189
Juvenile arthritis associated with increased symptomatic uveitis
Enthesitis related arthritis
190
Difference between Cytinosis and Cytinuria
Both have high cystiene but cytinuria= secretion in urine from cystinuria and cystiene deposition in cystinosis.
191
Where or what is the main problem in Cystienosis
Problem with cystiene transporter leading to accumulation. That's why deposition EVERYWHERE rather than just renal stones in cystinuria.
192
Which one causes Fanconi syndrome?
Cytinosis or Cystinuria
Cystinosis
